CEP290
适用: 小鼠
IF (cc), IF (p)
宿主: 兔
Polyclonal
AbBy Fluor® 750
应用备注
Optimal working dilution should be determined by the investigator.
限制
仅限研究用
溶解方式
Restore in distilled water.
缓冲液
0.1 M Tris, 0.1 M Glycine and 2 % Sucrose, None
注意事项
Avoid repeated freezing and thawing.
储存条件
4 °C/-20 °C
储存方法
Prior to reconstitution store the antibody at -20 °C. Store reconstituted antibody at 2-8 °C for one month or (in aliquots) at -20 °C for longer
抗原
CEP290
(Centrosomal Protein 290kDa (CEP290))
别名
CEP290
背景
CEP290 activates ATF4 mediated transcription and is required for the correct localization of ciliary and phototransduction proteins in retinal photoreceptor cells, may play a role in ciliary transport processes. CEP290 is ubiquitously expressed, strongly in placenta and weakly in brain. There are two named isoforms. Defects in CEP290 are a cause of Joubert syndrome type 5 (JBTS5) [MIM:610188], Senior-Loken syndrome type 6 (SLSN6) [MIM:610189], Leber congenital amaurosis type 10 (LCA10) [MIM:611755] and Meckel syndrome type 4 (MKS4) [MIM:611134]. Antibodies against CEP290 are present in sera from patients with cutaneous T cell lymphomas, but not in the healthy control population.Synonyms: CT87, Cancer/testis antigen 87, Centrosomal protein of 290 kDa, KIAA0373, NPHP6, Nephrocystin-6, Tumor antigen se2-2, se2-2